Systemic amyloidosis presenting as mucocutaneous bullous lesions

A 65-year-old male presented with hemorrhagic bullous skin lesions with purpura and ecchymoses. There was increased skin fragility with a strongly positive Nikolsky sign. Histopathology of the skin revealed large amounts of amyloid deposits in the dermis with a positive Congo Red staining around the dermal vessels. Examination and tests in this patient also revealed anemia, hepatomegaly, infiltrative cardiomyopathy, polyneuropathy and immunoglobulin λ deposition, favoring a diagnosis of primary amyloidosis (AL type). The present case is reported in view of the rarity of the bullous variant of primary systemic amyloidosis as well as presence of mucosal lesions and a positive Nikolsky sign.